Iniencephaly is a rare neural tube defect with characteristic retro-flexion of the fetal head and variable defect in the occipital bone. Pathogenesis is unknown. Crown-rump length is always small and discordant as compared to gestational age. Absence of cervicothoracic vertebrae. Associated occipital encephalocele can be seen. Associated spinal rachischisis can be seen. Prognosis is lethal. 

Ultrasound findings includes :

• "stargaze " appearance is the key to diagnosis
• hyperextension of the head